Hemorrhage Involving the Upper Airway in Hemophilia

Abstract

Bleeding involving the upper airway is a rare, highly emergent complication of hemophilia. This report describes the occurrence of two distinct episodes in a patient with severe factor IX deficiency. Clinical data from other cases described in the literature is summarized with regard to predisposing factors, age of incidence, presenting symptomatology, diagnosis, and management. Pertinent features of this bleeding complication include: high prevalence involving the pediatric population, presentation with non-specific symptoms (e.g., sore throat, dysphagia) early in the course of the bleeding episode, progression to complete upper respiratory obstruction if not recognized and treated, and the need for relatively high levels of the deficient coagulation factor for a period of at least 7 days to ensure resolution of the hematoma. The diagnosis is confirmed by observing retropharyngeal soft-tissue widening on lateral neck x-ray or alternatively, via cervical computed tomography.